| Main clinical features
|
severe fragility of skin and mucous membranes begining during delivery, large sheets of skin were detached, leaving superficial, not bleeding, dull, intense red wound surfaces
loss of profuse amounts of fluid from the extensive skin erosions, accompanied by universal alopecia and nail loss, and 3 triangular neonatal teeth ; death occurred at 10 days of age due to immense transcutaneous fluid loss
at histopathology of the skin, suprabasal clefting, leaving basal cells attached to the blister floor like a row of tombstones and at electron microscopy, disconnection of keratin intermediate filaments from desmosomes |